Notes 195-241

195. Saquinavir is an HIV protease inhibitor. Viral protease cleavage of a polyprotein is necessary for production of the viral coat protein.
The HIV protease inhibitors include ritonavir, indinavir, nelfinavir, and amprenavir in addition to saquinavir. Currently available HIV protease inhibitors are not sufficient for monotherapy because of the rapid emergence of resistance due to mutations in the viral protease. Combination therapy using saquinavir with two reverse transcriptase inhibitors such as zidovudine, lamivudine, abacavir, or didanosine is currently effective in reducing viral load.
Incorporation into RNA is part of the mechanism of action for the antitumor agent 5-fluorouracil (5-FU). Inhibition of thymidylate synthase is another part of the mechanism of action for the antitumor agent 5-FU. 5-FU is converted to 5-FdUMP, a potent inhibitor of thymidylate synthase.
Inhibition of viral DNA polymerase is a mechanism of action for the anti-herpesvirus nucleosides acyclovir, valacyclovir, famciclovir, and ganciclovir. These agents are metabolized to their nucleotide triphosphate forms, which competitively inhibit the herpesvirus polymerase. Acyclovir and valacyclovir also cause DNA chain termination.
Inhibition of viral reverse transcriptase is a property of the nucleosides zidovudine (AZT), didanosine (ddI), lamivudine (3TC), stavudine (d4T), and zalcitabine (ddC). These nucleosides are metabolized to the triphosphate forms that competitively inhibit reverse transcriptase.
Nonnucleoside reverse transcriptase inhibitors include nevirapine, delavirdine, and efavirenz. Combinations of agents are used because resistance, through mutations in the reverse transcriptase sequence, frequently arises during monotherapy.




196. In severely affected males with fragile X syndrome, amplifications are so great that restriction enzymes bracketing the triplet repeat region yield 500–2000 bp fragments that are easily dis- tinguished from normal sizes of 15–60 bp.
Analogous blotting techniques were named as puns on the name of Southern, including “Northern” blotting to detect RNA molecules and “Western” blotting to detect proteins. Immunoblotting is one version of the “Western” blot technique where proteins are separated by electrophoresis and transferred to a membrane for probing with antibody to the proteins of interest. Reverse transcription of RNA into single, then double-stranded DNA, followed by polymerase chain reaction amplification (RT-PCR), is a more sensitive method for RNA measurement. RT-PCR could demonstrate altered levels of fragile X mRNA but would not measure triplet repeats because these are outside of the fragile X coding region.



197. Patients may develop fever as a result of infectious or noninfectious diseases. The term fever of unknown origin (FUO) is applied when significant fever persists without a known cause after an adequate evaluation. Several studies have found the leading causes of FUO to include infections, malignancies, collagen vascular diseases, and granulomatous diseases. As the ability to more rapidly diagnose some of these diseases increases, their likelihood of causing undiagnosed persistent fever lessens. Infections such as intraabdominal abscesses, tuberculosis, hepatobiliary disease, endocarditis (especially if the patient had previously taken antibiotics), and osteomyelitis may cause FUO. In immunocompromised patients, such as those infected with HIV, a number of opportunistic infections or lymphomas may cause fever and escape early diagnosis. Self-limited infections such as influenza should not cause fever that persists for many weeks. Neoplastic diseases such as lymphomas and some solid tumors (e.g., hypernephroma and primary or metastatic disease of the liver) are associated with FUO. A number of collagen vascular diseases may cause FUO. Since conditions such as systemic lupus erythematosus are more easily diagnosed today, they are less frequent causes of this syndrome. Adult Still’s disease, however, is often difficult to diagnose. Other causes of FUO include granulomatous diseases (i.e., giant cell arteritis, regional enteritis, sarcoidosis, and granulomatous hepatitis), drug fever, and peripheral pulmonary emboli. Factitious fever is most common among young adults employed in health-related positions. A prior psychiatric history or multiple hospitalizations at other institutions may be clues to this condition. Such patients may induce infections by selfinjection of nonsterile material, with resultant multiple abscesses or polymicrobial infections. Alternatively, some patients may manipulate their thermometers. In these cases, a discrepancy between temperature and pulse or between oral temperature and witnessed rectal temperature will be observed.



198. The acute monoarticular arthritis in association with linear calcification of the cartilage of the knee suggests the diagnosis of pseudogout, also called calcium pyrophosphate dihydrate deposition disease. The disease resembles gout. Positive birefringent crystals (looking blue when parallel to the axis of the red compensator on a polarizing microscope) can be demonstrated in joint fluid. Serum uric acid and calcium levels are normal, as is the rheumatoid factor. Pseudogout is about half as common as gout but becomes more common after age 65. Calcium pyrophosphate dihydrate deposition disease is diagnosed in symptomatic patients by characteristic x-ray findings or crystals in synovial fluid. The disease is treated with NSAIDs or colchicine. Linear calcifications or chondrocalcinosis are often found in the joints of elderly patients who do not have symptomatic joint problems; such patients do not require treatment.


199. Behçet syndrome is a multisystem disorder that usually presents with recurrent oral and genital ulcers. Onefourth of patients develop superficial or deep vein thrombophlebitis. Iritis, uveitis, and nondeforming arthritis may also occur.



200. The 19-year-old with low back pain, morning stiffness, and eye pain has complaints that suggest ankylosing spondylitis. This is an inflammatory disorder that affects the axial skeleton. It is an autoimmune disorder that has a close association with HLA-B27 histocompatibility antigen. Anterior uveitis is the most common extraarticular complaint. Aortic regurgitation occurs in a few percent of patients.



201. Giant cell arteritis, also referred to as temporal arteritis or cranial arteritis, is a disease of elderly patients that classically affects the temporal arteries. Giant cell arteritis, named for the presence of giant cells and granulomata that disrupt the internal elastica of the vessel, may present with headache, anemia, a high ESR (although a normal ESR does not rule out the diagnosis), and occasionally a syndrome known as polymyalgia rheumatica. This includes stiffness, aching, and tenderness of the proximal muscles. These patients describe weakness of the hip and shoulder girdles, but there is no objective weakness of the muscles, and the muscle enzymes are normal. Giant cell arteritis usually responds to steroid therapy with 40 to 60 mg/d of prednisone; polymyalgia rheumatica typically responds to low-dose prednisone at 10 to 15 mg/d.



202. Reiter syndrome is a reactive polyarthritis that develops several weeks after an infection such as nongonococcal urethritis or gastrointestinal infection caused by Yersinia enterocolitica, Campylobacter jejuni, or Salmonella or Shigella species. Reiter syndrome is characterized as a triad of oligoarticular arthritis, conjunctivitis, and urethritis. The disease is most common among young men and is associated with the HLA-B27 locus. A circinate balanitis is painless and occurs in 25 to 40% of patients. Other clinical features may include waxy papules on the palms and soles called keratoderma blenorrhagicum, spondylitis, myocarditis, and thrombophlebitis. ANA and rheumatoid factor are usually negative. Gonorrhea can precipitate Reiter syndrome, but patients with the disease are culture negative.




203. Unilateral headache and visual loss in this elderly patient with polymyalgia rheumatica (PMR) symptoms lead to a clinical diagnosis of temporal arteritis. The erythrocyte sedimentation rate is high in almost all of these patients. Skull x-ray and CT scan would be normal. Carotid disease would not be expected. Temporal arteritis occurs most commonly in patients over the age of 55 and is highly associated with polymyalgia rheumatica. About 25% of patients with PMR have some features of giant cell arteritis. Thus, older patients who complain of diffuse myalgias and joint stiffness, particularly of the shoulders and hips, should be evaluated for PMR with ESR. Sudden visual loss in such a patient makes temporal arteritis an important diagnosis to make quickly.
PMR-Proximal muscle weakness of hip & shoulder.


204. The combination of fever, malar rash, and arthritis suggests systemic lupus erythematosus, and the patient’s thrombocytopenia, leukopenia, and positive antibody to native DNA provide more than four criteria for a definitive diagnosis. Other criteria for the diagnosis of lupus include discoid rash, photosensitivity, oral ulcers, serositis, renal disorders (proteinuria or cellular casts), and neurologic disorder (seizures). High-dose corticosteroids would therefore be indicated for any life-threatening complication of lupus such as glomerulonephritis, severe thrombocytopenia, or hemolytic anemia. Patients with SLE have an unpredictable course. Few patients develop all signs or symptoms. Neuropsychiatric disease occurs at some time in about half of all SLE patients, and Raynaud’s phenomenon in about 25%. Pregnancy is relatively safe in women with SLE who have controlled disease and are on less than 10 mg of prednisone.


205. The complaints (A 60-year-old female complains of dry mouth and a gritty sensation in her eyes. She states it is sometimes difficult to speak for more than a few minutes. There is no history of diabetes mellitus or neurologic disease. The patient is on no medications. On exam, the buccal mucosa appears dry and the salivary glands are enlarged bilaterally. The next step in evaluation is ?) described are characteristic of Sjögren syndrome, an autoimmune disease with presenting symptoms of dry eyes and dry mouth. The disease is caused by lymphocytic infiltration and destruction of lacrimal and salivary glands. Dry eyes can be measured objectively by the Schirmer test, which measures the amount of wetness of a piece of filter paper when exposed to the lower eyelid for 5 minutes. Most patients with Sjögren syndrome produce autoantibodies, particularly anti-Ro (SSA). Lip biopsy is needed only to evaluate uncertain cases, such as when dry mouth occurs without dry eye symptoms. Mumps can cause bilateral parotitis, but would not explain the patient’s dry eye syndrome. Corticosteroids are reserved for life-threatening vasculitis, particularly when renal or pulmonary disease is severe.



206. The clinical picture of symmetrical swelling and tenderness of the metacarpophalangeal (MCP) and wrist joints lasting longer than 6 weeks strongly suggests rheumatoid arthritis. Rheumatoid factor, an immunoglobulin directed against the Fc portion of IgG, is positive in about two-thirds of cases and is present early in the disease. The history of lethargy or fatigue is a common prodrome of RA. The inflammatory joint changes are not consistent with chronic fatigue syndrome. The MCP wrist distribution of joint symptoms makes osteoarthritis very unlikely. The x-ray changes described are characteristic of RA, but would occur later in the course of the disease.



207. The symptoms of Raynaud’s phenomenon, arthralgia, and dysphagia point toward the diagnosis of scleroderma. Scleroderma or systemic sclerosis is characterized by a systemic vasculopathy of small and medium-sized vessels, excessive collagen deposition in tissues, and an abnormal immune system. It is an uncommon multisystem disease affecting women more than men. There are two variants of scleroderma—a benign type called the CREST syndrome and a more severe diffuse disease. Antinucleolar antibody occurs in only 20 to 30% of patients with the disease, but a positive test is highly specific. Cardiac involvement may occur, and an ECG could show heart block or pericardial involvement. Renal failure can develop insidiously. Rheumatoid factor is nonspecific and present in 20% of patients with scleroderma.



208. Wegener’s granulomatosis is a granulomatous vasculitis of small arteries and veins that affects the lungs, sinuses, nasopharynx, and kidneys, where it causes a focal and segmental glomerulonephritis. Other organs can also be damaged, including the skin, eyes, and nervous system. Most patients with the disease develop antibodies to certain proteins in the cytoplasm of neutrophils called antineutrophil cytoplasmic antibodies (ANCA).



209. The 35-year-old with cough, sore throat, and fever went on to develop symptoms of myopericarditis with typical ECG findings. Coxsackievirus B infection is the most likely cause of URI symptoms that evolve into a picture of myocarditis. Myocarditis may be asymptomatic or can present with chest pain, both pleuritic and ischemic-like. Enteroviruses rarely if ever attack the pericardium alone without involving the subepicardial myocardium.



210. Gold therapy is still used in some patients with rheumatoid arthritis, especially in those who have not tolerated methotrexate. However, side effects are significant and include a dermatitis that may lead to exfoliative dermatitis if treatment is not discontinued, stomatitis, the nephrotic syndrome, and bone marrow suppression.



211. The rash of Rocky Mountain spotted fever (RMSF) occurs about 5 days into an illness characterized by fever, malaise, and headache. The rash may be macular or petechial, but almost always spreads from the ankles and wrists to the trunk. The disease is most common in spring and summer. North Carolina and East Tennessee have a relatively high index of disease. RMSF is a rickettsial disease with the tick as the vector. About 80% of patients will give a history of tick exposure. Doxycycline is considered the drug of choice, but chloramphenicol is preferred in pregnancy because of the effects of tetracycline on fetal bones and teeth. Overall mortality from the infection is now about 5%.



212. The patient has more than four of the required signs or symptoms of RA, including morning stiffness, swelling of the wrist or MCP, simultaneous swelling of joints on both sides of body, subcutaneous nodules, and positive rheumatoid factor. Subcutaneous nodules are a poor prognostic sign for the activity of the disease, and disease-modifying drugs (gold, penicillamine, antimalarials, or methotrexate) should be instituted. Methotrexate has emerged as the agent of choice. Oral corticosteroids are generally withheld unless absolutely necessary and after disease-modifying drugs are instituted. However, low-dose corticosteroids have recently been shown to reduce the progression of bony erosions. There is no value to using both aspirin and nonsteroidals together, as simultaneous usage will increase side effects.



213. Insidious back pain occurring in a young male that improves with exercise suggests one of the spondyloarthropathies—ankylosing spondylitis, Reiter syndrome, psoriatic arthritis, or enteropathic arthritis. Ankylosing spondylitis is most likely in this patient. Acute lumbosacral strain would not be relieved by exercise or worsened by rest. The prognosis in ankylosing spondylitis is generally very good, with only 6% dying of the disease itself. While pulmonary fibrosis and restrictive lung disease can occur, it is rarely a cause of death (cervical fracture, heart block, and amyloidosis are leading causes of death due to ankylosing spondylitis). Rheumatoid factor is negative in all the spondyloarthropathies. Crohn’s disease can cause an enteropathic arthritis, but this diagnosis is unlikely without gastrointestinal symptoms.



214. Felty syndrome consists of a triad of rheumatoid arthritis, splenomegaly, and leukopenia. In contrast to the lymphopenia observed in patients who have systemic lupus erythematosus, the leukopenia of Felty syndrome is related to a reduction in the number of circulating polymorphonuclear leukocytes. The mechanism of the granulocytopenia is poorly understood. Felty syndrome tends to occur in people who have had active rheumatoid arthritis for a prolonged period. These patients commonly have other systemic features of rheumatoid disease such as nodules, skin ulcerations, the sicca complex, peripheral sensory and motor neuropathy, and arteritic lesions



215. Polyarteritis nodosa- (Inflammation of small- to medium-sized muscular arteries, which may cause kidney, heart, liver, gastrointestinal, and muscular damage) is a multisystem necrotizing vasculitis that, prior to the use of steroids and cyclophosphamide, was uniformly fatal. In 30% of patients, antecedent hepatitis B virus infection can be demonstrated; immune complexes containing the virus have been found in such patients and are likely pathogenetic.




216. Howell Jolly bodies: eg post-spenlenectomy
Teardrop cells: Eg myelofibrosis and myelophthisis
Macro-ovalocytes: eg B12 deficiency
Target cells: If there is HbC or severe liver disease




217. The age and mediastinal location are typical for a lymphoblastic lymphoma involving the thymus. This lesion is in the spectrum of acute lymphoblastic !eukemia or lymphoma (ALL). Most ALL cases with lymphomatous presentation are of the pre-T cell type. This is supported by the expression of the T-cell markers CD2, CD5, and CD1. TdT is a marker of pre-T and pre-B cells. A Burkitt lymphoma is a B-cell lymphoma that may also be seen in adolescents but in the region of the jaw or abdomen. Nodular sclerosing Hodgkin disease does occur in the mediastinum, but it involves mediastinal nodes, not thymus. The histologic features of Hodgkin disease include the presence of Reed-Sternberg cells, and this variant has fibrous bands intersecting the lymphoid cells. Mantle cell lymphomas and follicular lymphomas are B-cell tumors usually seen in older patients, and they do not involve the thymus.



218. The features of gen. exfoliative erythroderma and skin biopsy showing lymphoid cells with cerebroform nucleii and them being present in blood too... implies Cutaneous T cell lymphoma... Sezary syndrome
Sezary syndrome (Cutaneous T-cell lymphoma) CD4 +ve and CD 8 -ve
A skin biopsy reveals the presence of lymphoid cells in the upper dermis and epidermis. These cells have cerebriform nuclei with marked infolding of nuclear membranes. Similar cells are seen on his peripheral blood smear.



219. Genes coding for LMP2, 3 and TAP 2 are present on chromosome: 6p
The MHC class II region also includes two alpha genes, DMA and DNA, and two beta genes, DMB and DOB, genes for the low-molecular-weight proteins (LMPs) LMP2 and LMP3 and for the transporter molecules TAP1 and TAP2.."


220. TNF alpha, beta genes are found in chromosome –6p
MHC class III region codes for complement components factor B, C2, and both C4 molecules, both tumor necrosis factor genes alpha and beta, and the heat shock proteins Hsp 1H and Hsp 70 2, and 21-hydroxylase.



221. Defect in Common variable immunodeficiency: Plasma cell absence
CVID is probably most common cause of pan-hypogammaglobulinemia in adults. Defect in terminal differentiation of B cells and hence absent plasma cells.



222.
a. Alpha heavy chain disease: Segilman disease
b. Gamma heavy chain disease: Franklin disease
c.Mu heavy chain disease is associated with CLL



223. HLA-A29 associated with birdshot retinochoroidopathy.

Rat bite lesions: punched out erosions with an overhanging rim of cortical bone. When these are adjacent to soft tissue tophi, they are pathognomonic of gout
Note: Meat, seafood are high purine
Milk, eggs are low purine
Note: Meat, seafood are high purine
Milk, eggs are low purine

But, most vegetables can be taken as low purine... EXCEPT : beans, peas, cauliflowers, mushrooms, spinach, lentils

In CPRS (complex regional pain syndrome/Reflex sympathetic dystrophy), swelling is diffuse in hands and NOT limited to joints. It is called catcher's mitt hand.


Leflunomide inhibits pyrimidine synthesis. It is carcinogenic, teratogenic, causes severe wt loss.


Still's disease = adult variant of JRA. Usually patients are 20-40 yrs old. Dramatic fever spiking upto 40degrees and then, fever may precede arthritis.
Also lymphadenopathy, pericardial effusions can happen.
An evanescent slamon-colored nonpruritic rash chiefly on the chest and abdomen is characterisitc... and it concides with fever spikes.



Note:
Cryoglobulins: main associations are Hep C and Sjogrens
Since 90% patients with cryoglobulinemia are associated with Hep C, Peg IFN alpha is standard care for mixed cryoglobulinemia
Cryoglobulins: types:
Type I -- (monoclonal proteins that lack rheumatoid factor activity) are more commonly seen in lymphoproliferative disease. (Type I cryoglobulins usually cause hyperviscosity syndromes rather than vasculitis.)
Type II --(monoclonal antibody with rheumatoid factor activity) can cause vasculitis
Type III- (polyclonal antibody with rheumatoid factor activity) cryoglobulins cause vasculitis.




Psoriatic arthritis: Assymetric sacroilitis + atypical syndesmophytes + relative lack of osteoporosis. Also pencil in cup deformity.



Causative agent for INDIAN tick typhus is : Rickettsia conorii


The main vector for rural malaria is: An. Culicifacies

(Height in cm)/(cube root of body wt in kg) is: Ponderal index



13.Which of these is correct:
a. National malaria eradication programme started in 1958
b. Modified Plan of Operation of NMEP started in 1977
c. Urban malaria scheme launched in 1971
d. All of the above


Lymph node of tongue: Jugulo-omohyoid
Lymph node of tonsil: Jugulo-digastric



Most commonly involved in raised ICT: Abducent
Most commonly involved in skull base #: Facial
Most commonly affected in spinal anesthesia: Abducent
Most thick cranial n: trigeminal
Most thin cranial n: trochlear



Note: Temperature receptors:
- Cold: End bulbs of Krause
- Warmth: Organs of Ruffini
- Heat: Organs of Golgi Mazzoni



Receptor for:
- Static balance: Macula
- Kinetic balance: Crista



Taste area (parietal operculum) in brain is: Area 43


Centre for extrapyramidal system' is: Area 6



a. Metacarpophalyngeal joints: Condylar joint
b. Interphalangeal joints: Hinge joints
c. First carpo-metacarpal joint: Saddle joint



Meckel cartilage: Malleus. ... So, Reichert cartilage : Stapes
Note: in ossicular chain embryology
Meckel cartilage (1st arch) gives malleus and incus
Reichert cartilage (2nd arch) gives stapes


Nerve of sixth arch is: Rec. laryngeal n
Note: Nerves of arches
1st: Mandibular div of CN5
2nd: CN7
3rd: CN9
4th: Sup laryngeal
5th: -
6th: Rec laryngeal




Note: larynx muscles in brief:

Adductors: (LTTE) . LTTE ppl close the glottis by adducting so that you cant breathe! How sick!
LTTE :
L - Lateral cricoarytenoids
T - Transverse arytenoids
T - Thyroarytenoids
E - c(e)ricothyroids (errr... c looks like e)


Abductors: (open glottis)
-Posterior cricoarytenoids


Tensors:
- Cricothyroids
- Thyroarytenoids


Relax vocal cords:
- Thyroarytenoids
- Vocalis



Close inlet of larynx:
- Oblique arytenoids
- Aryepiglotticus


Open inlet of larynx:
- Thyroepiglotticus



Main duct of pancreas: Wirsung
Accessory pancreatic duct: Santorini


Internal carotid artery passes through: Foramen lacerum


Nerve affected in Epicondylar Tunnel Syndrome: ulnar n
Note:
Epicondylar tunnel syndrome: Ulnar n at elbow
Carpal tunnel syndrome: Median
Tarsal tunnel syndrome: Posterior tibial n at ankle

Wrist drop: Radial n
Foot drop: deep peroneal n


Mutations in the BMPR2 gene cause pulmonary arterial hypertension.


Hering's N transmits impulses from carotid bodies to glossopharyngeal n
Note:
Arnold n / alderman nerve arises from vagus
Jacobson n arises from glossopharyngeal
Nervi erigentes are PARAsympathetic nerves



Narath's hernia is a femoral hernia associated with CDH
Note:
Laugier's femoral hernia: Hernia through gap in gimbernat's ligament
Narath's femoral hernia: Hernial sac lies between femoral vessels in Cong Dislocation of hip



224. Patients with classic Kawasaki disease must have 5 of the following symptoms, with fever an absolute criterion:
--Fever, lasting more than 5 days and refractory to appropriate antibiotic therapy
--Polymorphous erythematous rash
--Nonpurulent bilateral conjunctival injection
--Oropharyngeal changes, including diffuse hyperemia, strawberry tongue, and lip changes (eg,
swelling, fissuring, erythema, bleeding)
--Peripheral extremity changes, including erythema, edema, induration, and desquamation
--Nonpurulent cervical lymphadenopathy




225. Alkaline diuresis: Phenobarbital, Salicylate
Hemodialysis: Ethylene glycol, Lithium, Methanol, Salicylates, Theophylline
Hemoperfusion: Barbiturates, Theophylline

Further it says.. Charcoal hemoperfusion & hemodialysis have a role in barbiturate overdose for critical patients who do not respond to conservative therapy. Charcoal hemoperfusion is treatment of choice for Theophylline toxicity.


226. Piaget's theory of development of cognitive thinking in children encompasses four stages:
-Sensorimotor (18–24 months),
-Pre-operational (2 to 5–7 years),
-Concrete operational (6–11 years), and
-Formal operational (11 years to adulthood).


227. A young man is often the object of his friends’ jokes because he drops on the floor whenever he is having a good laugh. This young man suffers from :-
-Cataplexy refers to a sudden loss of muscle tone, ranging in severity from weakness in the knee to a total loss of tone, triggered by strong emotions, that takes place during full wakefulness.
-Cataplexy is thought to be due to an abnormal intrusion of REM sleep phenomena in periods of wakefulness.
-It is usually treated with medications that reduce REM sleep, such as antidepressants.
-Note that cataplexy can exist on its own... as well as a part of narcolepsy
-Then, also must not confuse cataplexy with catalepsy




228. A hemophilia patient with only 6% residual activity of factor 8 would be termed as:
Mild 6-30 %
Moderate 1-5 %
Severe <1 %




229.
ASD + PAPVR--- ASD likely to be sinus venosus defect
ASD + MR ----- ASD likely to be ostium primum defect
ASD + MS ----- ASD likely to be ostium secundum ., called Lutembacher syndrome






230. "Good ventricular function and low pulmonary vascular resistance are essential requirements for a successful Fontan procedure."
"The Fontan operation should not be performed when ventricular ejection fraction is less than 30% or ventricular end-diastolic pressure is greater than 15 mmHg"

Also note:
"Pulmonary vascular resistance in excess of 4 Woods units should also be considered an absolute contraindication for Fontan correction."
"Age at the time of Fontan procedure does not appear to be a major risk factor, provided age is greater than 2 years"




231. Defect in the following Proteins:--
MRP2- Dubin jonson
BSEP- Progressive familial intrahepatic cholestasis
CFTR- cystic fibrosis
F1C1 --Defect in Byler's disease is this protein




232. Which is the IgA type present in serum predominantly:
IgA1: primarily in serum
IgA2: more in secretions


233. Combined chemoradiation (the so-called Nigro protocol) promises to preserve continence, avoid colostomy, and offer a similar survival rate. For most lesions, chemoradiation—external-beam radiation, 5-fluorouracil, and mitomycin C—is the treatment of choice."




234.
Immunoglobulin Isotypes Mature B lymphocytes express IgM and IgD on their surfaces. They may differentiate by isotype switching (mediated by cytokines and CD40 ligand) into plasma cells that secrete IgA, IgE, or IgG.

IgG Main antibody in 2° response. Most abundant. Fixes complement, crosses the placenta,
opsonizes bacteria, neutralizes bacterial toxins and viruses.

IgA Prevents attachment of bacteria and viruses to mucous membranes, does not fix
complement. Monomer or dimer. Found in secretions. Picks up secretory component from
epithelial cells before secretion.

IgM Produced in the 1° response to an antigen. Fixes complement but does not cross the
placenta. Antigen receptor on the surface of B cells. Monomer or pentamer.

IgD Unclear function. Found on the surface of many B cells and in serum.

IgE Mediates immediate (type I) hypersensitivity by inducing the release of mediators from
mast cells and basophils when exposed to allergen. Mediates immunity to worms.
Lowest concentration in serum.



235. IL_1-------Fever
IL-2--------Stimulates T-Cells
IL-3--------Stimulates bone marrow.
IL-4--------Stimulates IgE production.
IL-5--------Stimulates IgA production.







236. The APGAR score (commonly performed at 1 and 5 minutes after birth; maximum
score is 10):

NUMBER OF POINTS GIVEN

CATEGORY 0 I 2

Heart rate Absent < 100 beats/rain > 100 beats/min
Respiratory effort None Slow, weak cry Good, strong cry
Muscle tone Limp Some flexion of extremities Active motion
Reflex irritability* None Grimace Grimace and strong cry, cough,
and sneeze
Color Pale. blue Body pink. extremities blue Completely pink

Reflex irritability usually is measured by the infant's response to stimulation of the sole of the foot or a catheter put inlo the nose.









237. Overdoses and antidotes

POISON OR MEDICATION ANTIDOTE

Acetaminophen
Cholinesterase inhibitors
Quinidine or tricyclic anti-depressants
Iron
Digoxin
Methanol/ethylene glycol
Benzodiazepines
Beta blockers
Lead
Copper or gold
Opioids
Carbon monoxide
Muscarinic blockers
Acetylcysteine
Atropine, pralidoxime
Sodium bicarbonate (cardioprotective)
Deferoxmnine
Normalize potassium and other electrolytes; digoxin Ab
Ethanol
Flumazenil
Glucagon
Edetate (EDTA); use succimer in children
Penicillamine
Naloxone
Oxygen (hyperbaric in cases of severe poisoning)
Physostigmine





238. IOLmaterial. Basically, IOLS can be divided into nonflexible and flexible types, as well as one-piece IOLs (in which the haptics and optic are made of a single material with no connecting points) and three-piece IOLs (in which the optic and haptics are made of different materials such as PMMA, polypropylene, and polyamide and connected to each other).

Nonflexible IOLs. These are mostly made of polymethylmethacrylate (PMMA). To implant a nonflexible IOL, the incision needs to be larger than the diameter of the IOL (5.5–6.5mm). Modern nonflexible IOLs are one-piece IOLs.

Flexible IOLs are folded with a forceps or an injector systemand are therefore implantable through 2.0–3.0mm incisions with the same optic size as non- flexible IOLs. Flexible IOLs are made of silicone, acrylic, hydrogel, or Collamer. The development andmodification ofmodern IOLs is a constantly ongoing process.

Cataract eyeglasses are only suitable for correcting bilateral aphakia. Cataract eyeglasses have the disadvantage of limiting the field of vision (peripheral and ring scotoma).




239. Kayser–Fleischer Ring: This golden-brown to yellowish-green corneal ring is caused by copper deposits at the level of Descemet’s membrane in Wilson’s disease (liver and lens degeneration with decreased serumlevels of ceruloplasmin). This ring is so characteristic that the ophthalmologist often is the first to diagnose this rare clinical syndrome.
In the early stages, a Kayser–Fleischer ring is best detected using gonioscopy.




240. Rigid Contact Lens: Previously, polymethylmethacrylate (PMMA) was used as a material. However, this is practically impermeable to oxygen. The lenses were fitted in small diameters with a very shallow curvature; the central area maintained contactwith the corneawhile the periphery projected. This allowed excellent tear film circulation, and patients were able to wear the lenses for surprisingly long periods.
Today, highly oxygen-permeable materials such as silicone copolymers are available. This eliminates the time limit for daily wearing. These lenses may also remain in the eye overnight in special cases, such as aphakic patients with poor coordination (prolonged wearing).

Rigid contact lenses can be manufactured as spherical lenses and toric lenses. Spherical contact lenses can almost completely compensate for corneal astigmatism of less than 2.5 diopters. This is possible because the space between the posterior surface of the spherical contact lens and the anterior surface of the astigmatic cornea is filled with tear fluid that forms a “tear lens.” Tear fluid has nearly the same refractive index as the cornea. More severe corneal astigmatism or internal astigmatism requires correction with toric contact lenses. Rigid contact lenses can even correct severe keratoconus.

Soft Contact Lenses
The material of the contact lens, such as hydrogel, is soft and pliable. Patients find these lenses significantly more comfortable. The oxygen permeability of the material depends on its water content, which may range from 36 to 85%. The higher the water content, the better the oxygen permeability. However, it is typically lower than that of rigid lenses. The material is more permeable to foreign substances, which can accumulate in it. At 12.5–16mm, flexible lenses are larger in diameter than rigid lenses. Flexible lenses are often supported by the limbus. The lens is often displaced only a few tenths of a milli-meter when the patient blinks. This greatly reduces the circulation of tear film under the lenses. This limits the maximum daily period that patients are able to wear them and requires that they be removed at night to allow regeneration of the cornea. Deviation from this principle is only possible in exceptional cases under the strict supervision of a physician.
As the lenses are almost completely in contact with the surface of the cornea, corneal astigmatism cannot be corrected with spherical soft lenses. This requires toric soft lenses.




241.Location of the extraocular muscle nuclei and gaze centers
Fig. 17.2 The oculomotor nerve (the third cranial nerve) supplies all of the extraocular muscles except the superior oblique (which is supplied by the trochlear nerve/fourth cranial nerve) and the lateral rectus (supplied by the abducent nerve/sixth cranial nerve). The rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) is responsible for vertical eye movement and phases of rapid nystagmus. The paramedian pontine reticular formation (PPRF) is responsible for horizontal eye movement.